Von Willebrand Disease Flashcards
(11 cards)
How does Von Willebrand disease affect the clotting screen?
Isolated rise in PT and reduction in ACTIVITY of factor VIII
What are the types of vWF disease?
Type 1 VWD: Partial quantitative deficiency in VWF
Type 2 VWD: Qualitative defects in VWF (e.g. decreased adhesion to platelets or factor VIII)
Type 3 VWD: Almost complete deficiency of VWF
What is the first line treatment for VWF?
First line medication is Desmopressin which works by temporarily increasing FVIII and VWF levels by releasing endothelial stores
What is given for acute bleeding episodes in VWF disease?
Desmopressin should be administered if not already being taken
Tranexamic acid can be given for minor bleeding or prior to surgery on its own or as an adjunctive therapy to desmopressin or concentrates
VWF-FVIII concentrates should be used if the above are unsuccessful and bleeding is persistent
What is the intrinsic pathway?
Factor 12
Factor 11
Factor 9
Factor 8
What is the extrinsic pathway?
Factor 7 and Ca2+ and factor 3
What causes activation of factor X?
Phospholipids
What is the epidemiology of vWF?
Most common inherited bleeding disorder affecting pTH men and women equally
How is platelet count affected n vWF disease?
No change
-> it is a functional platelet disorder
What are the coagulation findings for vWF?
Normal PT time
Long PTT time
Factor VIII activity decreased
How is platelet count affected in vWF?
No change
